主任查房-武金城-49M--5-教学课件

主任查房主任查房武金城49/M2019-5-16病例特点病例特点l中年男性，隐袭起病，慢性病程中年男性，隐袭起病，慢性病程l肾脏受累肾脏受累大量蛋白尿、低白蛋白血症、肾功能受损l糖尿病、糖尿病、非活动性非活动性HBsAg携带携带诊断与鉴别诊断ARF or A/C急性肾衰竭急性肾衰竭肾前性肾衰竭肾前性肾衰竭肾性肾衰竭肾性肾衰竭肾后性肾衰竭肾后性肾衰竭肾小球性肾小球性肾小管性肾小管性肾间质性肾间质性肾血管性肾血管性无证据无证据肾小球：急进性肾小球肾炎急进性肾小球肾炎l支持：蛋白尿支持：蛋白尿lANAANA、ANCA-PR3 ANCA-PR3（-）；无明显血尿无明显血尿肾间质：急性间质性肾炎、特发性急肾衰急性间质性肾炎、特发性急肾衰l支持点：有可疑用药史、蛋白尿、肾小支持点：有可疑用药史、蛋白尿、肾小管损伤证据管损伤证据l不支持点：不支持点：无相应体征、血嗜酸细胞正无相应体征、血嗜酸细胞正常常、蛋白尿量大、蛋白尿量大肾小管：急性肾小管坏死急性肾小管坏死支持：用药史、有支持：用药史、有近端肾小管重吸收功能近端肾小管重吸收功能障碍障碍不支持：不支持：无明显少尿、无尿及多尿的病程，无明显少尿、无尿及多尿的病程，低比重尿低比重尿肾血管l肾动脉狭窄、血栓肾动脉狭窄、血栓l肾静脉血栓肾静脉血栓l血栓性微血管病血栓性微血管病肾脏本病肾脏本病的诊断的诊断l大量蛋白尿大量蛋白尿(尿蛋白尿蛋白3.5/)l低蛋白血症低蛋白血症(血浆白蛋白血浆白蛋白30%reduction in GFR within 2-3 months after initiation of an ACE inhibitor or ARB.AJKD,VOL49,NO2,SUPPL2,FEBRUARY2019kidney.org/professionals/kdoqi/guideline_diabetes/guide1.htm其他l糖尿病病史短l尿蛋白分子量测定特点肾脏病理MembranousNephropathy（MN）ldistinctclinicopathologicentitybyDavidJonesin1957Nephroticglomerulonephritis.AmJPathol.1957;33:313-329.lCirculatingautoantibodies(immunoglobulinG4andG1subclasses)interactingwithantigensnativetoorplantedintheGlomerularcapillarywallatthepodocytecellmembranebasementmembraneInterfacegenerallyareregardedasthefundamentalpathobiologicalmechanismlMNnowisregardedasapodocytopathyamericanJournalofKidneyDiseases,Vol56,No1(July),2019:pp157-167IgGsubclasseslnamedaccordingtotheirabundanceintheserum:lIgG1comprises60to70percentoftotalIgGlIgG2comprises20to30percentlIgG3comprises5to8percentlIgG4comprises1to4percentSerumIgGsubclassesinnormaladults.MonogrAllergy1986;19:100.DoiT，MayumiM，KanatsuK，etalDistributionofIgGsubclassesinmembranousnephropathyClinExplmmunol，1984，58：57-62IgG1、IgG3通过Th1细胞分泌的细胞因子IL-2、IFN-r、TNF-发挥细胞毒作用IgG1andIgG3fixC1qmosteffectivelywhileIgG2doessoweakly.IgG4通过Th2系统激活B细胞参与免疫反应IgG4doesnotbindcomplementatallIgGsubclassrestriction(antibodyresponsestocertaintypesofpathogens):lbacterialpolysaccharideantigens,suchasthecapsularantigensofpneumococcus,arecomposedmainlyofIgG2moleculeslproteinantigensandviralantigensarecomposedpredominantlyofIgG1andIgG3lIgG4incompletelyunderstood,althoughthefindingthatantibodyresponsestochronicschistosomiasisandfilariasesareprimarilycomposedofIgG4moleculessuggeststhatIgG4mayplayaroleintheimmuneresponseagainstparasiticdiseaseIMN患者肾组织中仅患者肾组织中仅IgG4沉积或沉积或IgG4沉积为主，沉积为主，可伴有可伴有IgG1沉积沉积1.ImaiH，HamaiK，KomatsudaA，etalIgGsubclassesinpatientswithmembranoproliferativeglomerulonephritis，membranousnephropathy，andlupusnephritisKidneyInt，2019，51：270-2762.KurokiA，ShibataT，HondaH，etalGlomerularandserumIgGsubclassesindiffuseproliferativelupusnephritis，membranouslupusnephritis，andidiopathicmembranousnephropathyInternMed，2019，41：936-942提示提示IMN可能与多条免疫反应途径有关，但以可能与多条免疫反应途径有关，但以Th2介导的体液免疫反应为主介导的体液免疫反应为主M-TypePhospholipaseA2ReceptorasTargetAntigeninIdiopathicMNlM-PLA2R抗体抗体-adult IMN antigenl存在于正常足细胞表面的膜蛋白存在于正常足细胞表面的膜蛋白，其在肾组其在肾组织有高水平表达。织有高水平表达。l大多数特发性膜性肾病患者有抗大多数特发性膜性肾病患者有抗PLA2R构构象依赖性表位的抗体象依赖性表位的抗体，是该病一种主要抗，是该病一种主要抗原原。M-typephospholipaseA2receptorastargetantigeninidiopathicmembranousnephropathyJNEngIJMed，2009，361(7)：11-21IMN患者血清中的抗患者血清中的抗PLA2R自身抗体主要为自身抗体主要为IgG4亚型亚型M-typephospholipaseA2receptorastargetantigeninidiopathicmembranousnephropathyJNEngIJMed，2009，361(7)：11-21病理上DKD与MN区分lFMl抗体种类、沉积成分l沉积部位l荧光图像lLMlEM亚急性小管间质病IgG4-relatedsystemicdiseaseProposalfordiagnosticcriteriaforIgG4-relatedkidneydiseaselBetween2019and2019,weidentified41patientswithIgG4-RKDin5UniversityHospitalClinExpNephrol(2019)15:615626lIgG4-associatedidiopathictubulointerstitialnephritiscomplicatingautoimmunepancreatitisNephrolDialTransplant(2019)19:474476lIgG4-relatedChronicTubulointerstitialNephritiswithoutAutoimmunePancreatitisandtheTimeCourseofRenalFunctionInterMed49:1593-1598,2019CharacteristictubulointerstitialnephritisinIgG4-relateddiseaselwell-demarcatedbordersbetweeninvolvedanduninvolvedareas;HumanPathology(2019)43,536549linvolvementofthecortexandmedulla,oftenextendingbeyondtherenalcapsuleandwithoccasionalextensiontoretroperitonealfibrosis;lInterstitialinflammatorycellscomprisingpredominantlyplasmacellsandlymphocytes,withahighprevalenceofIgG4-positivecellsoftenadmixedwithfibrosis;lpeculiarfeaturesofInterstitialfibrosisresemblinga“birds-eye”patterncomprisingfibrosisamonginterplasmacellspaces;lDepositsvisiblebylightandimmunofluorescentmicroscopyinthetubularbasementmembrane,Bowmancapsule,andinterstitiumthatarerestrictedtotheinvolvedportion,sparingnormalparts.lMembranoproliferativeGlomerulonephritis-LikeGlomerularDiseaseandConcurrentTubulointerstitialNephritisComplicatingIgG4-RelatedAutoimmunePancreatitisInterMed48:157-162,2009lIgG4-RelatedTubulointerstitialNephritisWithMembranousNephropathyAmJKidneyDis.2019;58(2):320-324DiagnosisofAutoimmunePancreatitis:TheMayoClinicExperiencelOnthebasisofthisexperienceweproposethatdiagnosisofAIPcanbemadeinpatientswith1ofthesecriteria:(1)diagnostichistology,(2)characteristicimagingoncomputedtomographyandpancreatographywithelevatedserumIgG4level,or(3)responsetosteroidtherapyofpancreatic/extrapancreaticmanifestationsofAIP.Clinical Gastroenterology and Hepatology Volume 4,Issue 8,August 2019,Pages 10101016目前诊断目前诊断l肾病综合征结节型糖尿病肾小球硬化症伴膜性肾病亚急性肾小管间质病变代谢性酸中毒l2型糖尿病糖尿病视网膜病变（增殖期）l高血压病（3级，极高危）l非活动性HBsAg携带目前治疗目前治疗查房目的l明确诊断明确诊断l指导下一步诊治指导下一步诊治l教学查房教学查房